Arteriovenous (AV) fistulas are common in renal cell carcinoma. These are usually small and asymptomatic. Case reports have previously described symptomatic fistulas in primary renal cell tumour or, less commonly, bone metastases. The current study describes a patient with lung metastases from renal cell carcinoma who developed a symptomatic pulmonary AV fistula. The patient presented with debilitating progressive shortness of breath, which resolved after coiling of the pulmonary AV fistula. Supporting radiographs include a unique ventilation-perfusion finding of perfusion tracer in the kidney that is diagnostic of a pulmonary shunt. This is the first report of a pulmonary fistula from renal cell carcinoma. This rare complication of renal cell carcinoma reflects its diverse clinical presentations and unique tumour biology.